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Kie Honjo, MD; Tomohiko Ohshita, MD, PhD; Hideshi Kawakami, MD, PhD; Hiromitsu Naka, MD, PhD; Yukari Imon, MD, PhD; Hirofumi Maruyama, MD; Yasuyo Mimori, MD, PhD; Masayasu Matsumoto, MD, PhD

Arch Neurol. 2004;61:933-937.

Background  Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant cerebellar ataxia caused by CAG trinucleotide expansion. The characteristics of regional cerebral blood flow (rCBF) in SCA6 patients have not been established, whereas it has been reported that decreased rCBF in the cerebrum seems to be a remote effect of cerebellar impairment in other cerebellar disorders.

Objective  To clarify the characteristics of rCBF, including cerebro-cerebellar relationship, and its correlation with clinical manifestations in patients with genetically confirmed SCA6 using quantitative assessment of rCBF by brain single-photon emission computed tomography (SPECT).

Design  Technetium Tc 99m ethyl cysteinate dimer SPECT study using a Patlak plot.

Patients  Hiroshima University Hospital, Hiroshima, Japan. Ten patients with SCA6 and 9 healthy controls.

Main Outcome Measure  The rCBF of the cerebellar vermis, cerebellar hemisphere, and frontal lobes.

Results  In SCA6 patients, rCBF was decreased only in the cerebellar vermis and hemisphere compared with healthy controls, and this was inversely correlated with duration of illness. The rCBF in the frontal lobes was slightly correlated with duration of illness without statistical significance. The rCBF in the vermis was inversely correlated with severity of dysarthria, but there was no significant correlation with CAG repeated expansions.

Conclusions  Decrease in rCBF was found only in the cerebellum and was associated with duration of illness, dysarthria and ataxia, and cerebellar atrophy. No remote effect of cerebellar hypoperfusion was found in the SCA6 patients.

From the Department of Clinical Neuroscience and Therapeutics, Hiroshima University, Graduate School of Biomedical Sciences (Drs Honjo, Ohshita, Kawakami, Imon, Maruyama, Mimori, and Matsumoto), and Department of Neurology, Suiseikai Kajikawa Hospital (Dr Naka), Hiroshima, Japan.

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